Christmas Disease: The Incredible Impact of Hemophilia B on European Royalty

Hemophilia B, aka Christmas Disease, is a Rare Condition with a Fascinating Story – One That Helped Shape European History

Dan
Dan
December 6, 2018
Christmas/Royal Disease title image: King's Church, Windsor

Christmas Disease is one of the aliases of a rare bleeding disorder called Hemophilia B. Its other pseudonym, Royal Disease, hints at its colorful history and the surprising impact it has had on monarchies ranging from the UK to Russia.


What is Christmas Disease?

While Christmas fever may be an affliction that results in a certain Mariah Carey song being overplayed for weeks on end, Christmas Disease is an altogether more serious matter.

Christmas disease is, in fact, the alias of Hemophilia B. Hemophilia B is a rare bleeding disorder caused by a deficiency of a protein called Factor IX (F9) – one of the 12 Factors involved in blood clotting. As a result, those living with it – usually males – tend to bleed longer than would be considered normal.

Hemophilia B affects the X chromosome. Because males have one X chromosome, inherited from their mother, and one Y chromosome, inherited from their father, sons can only inherit the faulty gene from their mother. If they do, they will suffer from Hemophilia B.

Females who inherit one faulty gene, however, are likely to inherit a healthy one as well which counteracts it. This means that, while they will be a carrier, they will not experience symptoms. Of course, should a female inherit two faulty genes, they too will suffer from the disease, but this can only happen if the father has Hemophilia B and the mother is a carrier. Hemophilia B is therefore very rare in females.

Those living with mild Hemophilia B (about 25% of cases) have F9 levels between 6-30% and may suffer excessive bleeding after serious trauma or surgery. Women with mild Hemophilia B are likely to experience menorrhagia – heavy menstrual bleeding – and postpartum bleeding.

Those with moderate Hemophilia B (about 15% of cases) have F9 levels between 1-5%. In these cases, bleeding may occur without an identifiable cause, a phenomenon known as spontaneous bleeding. The bleeding comes from deep tissue and can also occur after an injury or surgery. If left untreated, it can cause permanent damage in areas such as joints.

In severe cases, when F9 levels are below 1%, such spontaneous bleeding can happen once or twice per week.

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Why is Hemophilia B Called Christmas Disease?

Despite its merry-sounding name, Christmas Disease has nothing to do with the festive season. In fact, it is named after Stephen Christmas, the five-year-old boy who was the first patient described in a paper outlining the distinct characteristics of Hemophilia B.

It was noted in the literature, published by Biggs et al, that young Christmas and other patients examined, did not lack the ‘antihemophilic globulin’ and thus did not have regular hemophilia. However, the symptoms and length of time blood took to clot meant they would usually be diagnosed as such.

The antihemophilic globulin to which the researchers refer to is now recognized as Factor XIII (F8), a deficiency of which is referred to these days as Hemophilia A – the more common form of hemophilia. Around one in every 4,000-5,000 males born has Hemophilia A, compared to one in every 20,000 being born with Hemophilia B.

The discovery that there were two forms of Hemophilia, A and B, had actually been made five years earlier by Argentinian physician, Alfredo Pavlovsky.

Throughout the ’50s and ’60s, cases such as Christmas’ helped define the different conditions and the factors involved in blood coagulation. Since then, effective treatment has been developed and Hemophilia B is far from the fatal disease it once was.

If we rewind to the 19th Century, however, when Hemophilia B was a far deadlier disease, we come across a remarkable set of events that had a massive impact on European history. These events are also why the disease is known by another name: The Royal Disease.

Why is Hemophilia B Also Called Royal Disease?

It all started with one of the most famous British monarchs, Queen Victoria, who was born in 1819. Queen Victoria was a carrier of Hemophilia B. This fact alone was enough for British author, A. N. Wilson, to question her legitimacy – a matter that has long been subject to rumor.

Victoria’s father, Prince Edward, Duke of Kent, did not have the disease himself, nor is there any evidence of the condition in the ancestry of Victoria’s mother, the Duchess of Kent. The theory, therefore, is that Prince Edward was not her father at all.

A logical rebuttal to this is that the would-be illegitimate father – rumored to be a soldier named Sir John Conroy – must have had the disease himself. Given the fact that Conroy lived to the ripe old age of 67, far beyond the life expectancy of 11 years old those with Hemophilia had in those days, this seems somewhat unlikely.

It is possible that Victoria’s development of the Hemophilia B gene was a spontaneous mutation, which happens in 30% of cases. It is a mystery that may remain unsolved unless the royal family gives unprecedented permission for genetic testing to be carried out. Regardless, the impact Hemophilia B was to have transcended borders and generations alike.

As Victoria was a carrier, any sons she mothered would have a 50% chance of developing Hemophilia B and any daughters would have an equal chance of becoming carriers. How many children did Queen Victoria and her husband, Prince Albert, have?

Nine.

Of their four sons, only Prince Leopold inherited the gene and developed Hemophilia B. Of their five daughters, Princess Alice and Princess Beatrice inherited the gene and became carriers. It is possible other daughters also became carriers but never passed the gene along to their sons.

Queen Victoria was of the opinion that intermarriage between European royalty could ensure lasting peace. She subsequently became something of a matchmaker for her nine children and 42 grandchildren, weaving a web of royal intermarriages in which Hemophilia B was intrinsically tangled.


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The Historical Impact of the Royal Disease – Hemophilia B

Prince Leopold, Duke of Albany

Prince Leopold’s story is the most straight-forward. He died in 1884, at 30 years of age, one year after the birth of his daughter, Princess Alice. He died after suffering a head injury that led to cerebral hemorrhaging, an injury he would have perhaps survived were it not for the bleeding – or, rather, the lack of blood clotting – caused by Hemophilia B.

Princess Alice was a carrier of the disease – as all daughters of those with it are. Of her three children, it was only her youngest son, Prince Rupert, who inherited the gene. He died in a car crash at the age of 20, bringing an end to Hemophilia B in that particular lineage.

Princess Beatrice & Hemophilia B’s Impact on the Spanish Monarchy

Things are rather more interesting when it comes to Princess Beatrice, who fell in love with Prince Henry of the Battenberg family. Queen Victoria eventually consented to their marriage having initially opposed it, on the condition that Henry relinquished his German commitments and moved permanently to England.

Of the four children Beatrice and Henry had before the latter died ten years after their marriage, in 1896, the Hemophilia B gene was passed down to at least two, perhaps three.

Hemophilia B likely played a role in the death of one son, Prince Leopold of Battenberg, who died following an operation aged 32. Another son, Prince Maurice, may or may not have had the condition, and was killed in action in World War I.

Most significantly, the gene was passed on to Princess Victoria Eugenie, better known in Britain as Ena. Through her marriage to King Alfonso XIII, Hemophilia B made its way into the Spanish royal family. Although Hemophilia was not completely understood back then, Alfonso XIII has been aware there was a possibility that Victoria Eugenie was a carrier of a blood disorder. It was one of the reasons his mother opposed the marriage. Despite these concerns, the wedding went ahead in Madrid in 1906.

Unfortunately, their first son, Infante (Prince) Alfonso, Prince of Asturias, was confirmed to have the disease as a baby when his bleeding would not stop following circumcision. Their fourth son, Infante Gonzalo, is also believed to have had the disease and died at the age of 20 following a car crash. He hadn’t appeared to suffer severe injuries at first but was killed by the bleeding that resulted.

In between, their second son, Infante Jaime was deaf, leaving their third son, Infante Juan as the only of the four sons who could succeed King Alfonso XIII.

Alfonso XIII never forgave Beatrice for passing the disease on to their sons and it severely damaged their relationship. And while his reign was a tumultuous one in general, the health issues surely weakened the position of the monarchy in the eyes of the public.

Alfonso XIII fled Spain in 1931 and the Spanish monarchy was abolished. The series of events that followed, in particular the Spanish Civil War 1936-39 that left hundreds of thousands of people dead, lead to Francisco Franco assuming power. General Franco’s subsequent leadership, which lasted until 1975, was that of a ruthless dictator who sympathized with the likes of Hitler and Mussolini.

Shortly before Franco’s death in 1975, he reinstated the monarchy. Alfonso XIII’s grandson, Juan Carlos I, took the throne.

It is hard to say just how much of an impact Hemophilia B had on these events, but the fact that two of King Alfonso XIII’s sons suffered from the condition undoubtedly had a huge impact on his life and his relationship with Queen Beatrice. Such tragedies must also have played a part when it came to public opinion of and support for the monarchy.

What might have happened had the coin had landed the other way for Infante Alfonso and Infante Gonzalo, and they hadn’t inherited the Hemophilia B gene, is no more than an interesting topic of discussion. It seems likely, however, that one of the most significant periods of recent European history would have played out rather differently.

Princess Alice & Hemophilia B’s Impact on the Russian Monarchy

While Beatrice’s marriage may have had a profound effect on Spanish history, Princess Alice’s headed in another direction: Russia.

Alice married Prince Louis of Hesse. Together they had seven children before Alice died aged 35 of diphtheria, which also afflicted Louis and six of their children. Their youngest child, Princess Marie, had died from the disease in 1878, aged just 4.

Regarding Hemophilia B, at least three of Alice and Louis’ children inherited the gene: Prince Friedrich, Princess Irene, and Princess Alix. Tragically, it claimed the life of Prince Friedrich before his third birthday, after he fell from a window. He subsequently died of brain hemorrhaging.

There were also tragic consequences for Princess Irene. Following Irene’s marriage to Prince Henry of Prussia, two of their three sons inherited the disease. While Prince Waldemar survived to the age of 56, Prince Heinrich died aged 4. As in the case of Friedrich, Heinrich’s death was the result of a fall, and may have been avoided were it not for Hemophilia B.

However, it is the story of Princess Alix that has the most historical significance.

On November 26, 1894, she married Nicholas II of the House of Romanov, the dynasty that had ruled Russia since 1613. On the 1st of that month, Nicholas II had become the Emperor of Russia (Tsar) following the death of his father, Alexander III. Princess Alix took the name Alexandra Feodorovna.

Alexandra was not a popular Empress (Tsaritsa/Tsarina) in Russia. She was German-born which, given the rather tense relations between the countries at the time, meant she was met with widespread suspicion.

Four days after their official coronation as Emperor and Empress, in 1896, a banquet was held for the people on Khodynka Field in Moscow. Thousands turned up, expecting food and beer. When rumors began circulating that there was not enough prezels and beer for everyone, a crush ensued. Over 1,300 people were killed.

Nicholas II and Alexandra’s decisions to attend a ball held at the French embassy that night, and to hold a day of mourning following the death of Archduke Karl Ludwig of Austria earlier in the month but not the 1,300 Russians killed in the crush, were not popular with the people.

The fact Alexandra’s first four children were girls did little to help her cause, desperate as the people were for a male heir. She finally produced one, Tsarevna Alexei, in 1904.

Alexei was born with severe Hemophilia B.

His condition was kept secret, for Tsars, “as head of the Church and leader of the people, must be free of any physical defect.”

Throughout the course of Alexei’s childhood, Alexandra grew fond of faith-healer/con-man (depending on one’s perspective), Grigori Rasputin. Alexandra believed Rasputin had improved Alexei’s health on several occasions, and he became an influential figure within the family.

Rasputin became a deeply unpopular figure among the Russian people. His influence over the royal family, particularly Alexandra, was used to discredit them, especially after the start of World War I. Rasputin was assassinated in December 1916.

The following year, the two-part Russian Revolution occurred. The first part – the February Revolution – ended with the fall of the Imperial Government when Nicholas II abdicated, bringing an end to the 300-year-long dynasty.

The second part of the revolution in October, known as Red October, the October Uprising, and the Bolshevik Revolution, ended in victory for the Bolshevik Party, led by Vladimir Lenin. It also marked the beginning of the five-year-long Russian Civil War.

In 1918, Nicholas II, Alexandra, and their entire family were executed.

Were it not for Alexei inheriting Hemophilia B, Alexandra and Nicholas II may well have had a healthy heir, Rasputin may not have gained influence, public opinion of the Romanov family may have been considerably better, and the Bolshevik Revolution may never have occurred.

As with the events that happened in Spain, of course, this is purely conjecture.

Nonetheless, it is fair to say that for a disease as rare as Hemophilia B, it has had quite the impact on European history.

Hemophilia B Today

Today, there is only a small chance that Hemophilia B is carried within European royal families. Should it occur once more, however, it is unlikely to have the impact it once did.

While it is still a serious condition, especially in severe cases, treatments such as replacement therapy help those diagnosed with the condition live healthily. Replacement therapy, which involves regular IV injections of F9 (or F8 in the case of Hemophilia A), may soon be replaced by gene therapy.

It can be hoped, therefore, that the devastation caused by the disease over the centuries is now safely confined to the annals of history.


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